Situs inversus totalis and secondary biliary cirrhosis: a case report

Situs inversus totalis (SIT) is a congenital anomaly characterized by complete transposition of abdominal and thoracic organs. As a birth defect in newborn infants, it has an estimated incidence of 1/15000 to 10000 cases in live births, with a male/female ratio of 3:2. Generally, this rare anomaly is diagnosed incidentally during thoracic and abdominal imaging. The cause of situs inversus (SI) is unknown. More than one genetic mutations including gene mutations which cause ciliopathy and cystic renal diseases were implicated in etiopathogenesis [1]. SIT is associated with various gastrointestinal abnormalities. In the current literature, development of intestinal ischemia due to intestinal malrotation, and also acute appendicitis and liver transplantation due to juvenile biliary atresia were reported [2-4]. However, there is no data for the development of secondary biliary cirrhosis (SBC) due to extrahepatic cholestasis in a patient with SIT. We here presented a case of SIT with SBC who referred to our clinic due to extrahepatic cholestasis.A 58-year-old female patient, who complained of icterus appearing in the last 6-7 months, along with the symptoms of fatigue and loss of appetite continued for 2-3 years, was referred to our clinic. According to her medical history, she had been referred to a clinic because of abdominal pain in the left lower quadrant and examined due to acute abdominal pain when she was 6 years old. She had undergone a surgical operation due to acute appendicitis located in the left lower quadrant and the SIT was diagnosed on those days. Furthermore, frequently recurrent upper respiratory tract infections, hypertension and a previous cholecystectomy (19 years ago) were found in her medical history. The patient was a smoker (26 packs/year) but she did not consume alcohol. In detailed personal history, she did not have any hepatotoxic drug usage in past three months. In her physical examination, icteric appearance, moderate hepatomegaly and kyphos